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Supporting information S1: PARKINSON PLUS SCALE (NNIPPS-PPS) (83 items)
NNIPPS-PPS 1/6
MENTAL FUNCTION (interview with patient or caregiver)
1. Intellectual impairment
6. Loss of concentration / Integration ability
0 None.
0 Clearly absent.
1 Mild. Consistent forgetfulness with partial recollection of events 1 Equivocal or minimal.
and no other difficulties.
2 Clearly present, but not interfering with activities of daily living
2 Moderate memory loss, with disorientation and moderate
(ADL).
difficulty in handling complex problems. Mild but definite
3 Interfering mildly with ADL.
impairment of function at home with need of occasional
4 Interfering markedly with ADL.
prompting.
3 Severe memory loss with disorientation for time and often to
7. Bradyphrenia
place. Severe impairment in handling problems.
0 Clearly absent.
4 Severe memory loss with orientation preserved to person only. 1 Equivocal or minimal.
Unable to make judgements or solve problems. Require much help2 Clearly present, but not interfering with activities of daily living
with personal care. Cannot be left alone at all.
(ADL).
3 Interfering mildly with ADL.
2. Thought disorder
4 Interfering markedly with ADL.
0 None.
1 Vivid dreaming.
8. Emotional incontinence
2 « Benign » hallucination with insight retained.
0 Clearly absent.
3 Occasional to frequent hallucinations or delusions without
1 Equivocal or minimal.
insight; could interfere with daily activities.
2 Clearly present, but not interfering with activities of daily living
4 Persistent hallucinations, delusions, or florid psychosis. Not able (ADL).
to care for self.
3 Interfering mildly with ADL.
4 Interfering markedly with ADL.
3. Depression
0 Not present.
1 Periods of sadness or guilt greater than normal, never sustained
for days or weeks.
2 Sustained depression (1 week or more).
3 Sustained depression with vegetative symptoms (insomnia,
anorexia, weight loss, loss of interest).
4 Sustained depression with vegetative symptoms and suicidal
thoughts or intent.
9. Withdrawal
0 Clearly absent.
1 Equivocal or minimal.
2 Clearly present, but not interfering with activities of daily living
(ADL).
3 Interfering mildly with ADL.
4 Interfering markedly with ADL.
10. Grasping / imitative / Utilising behaviour
4. Motivation / Initiative
0 Clearly absent.
0 Normal.
1 Equivocal or minimal.
1 Less assertive than usual, more passive.
2 Loss of initiative or disinterest in elective (non-routine) activities.2 Clearly present, but not interfering with activities of daily living
(ADL).
3 Loss of initiative or disinterest in day-to-day (routine) activities.
3 Interfering mildly with ADL.
4 Withdrawn, complete loss of motivation.
4 Interfering markedly with ADL.
5. Aggressiveness
0 No increase in aggressiveness.
1 Increased, but not interfering with family interactions.
2 Interfering with family interactions.
NNIPPS - Parkinson Plus Scale – Supplemental information final 16.02.2011
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NNIPPS-PPS 2/6
BULBAR-PSEUDOBULBAR SIGNS
1. Salivation
4. Facial expression
0 Normal.
0 Normal.
1 Slight but definite excess of saliva in mouth; may have night-time1 Minimal hypomimia, could be normal « Poker Face ».
drooling.
2 Slight but definitely abnormal diminution of facial expression.
2 Moderately excessive saliva; may have minimal drooling.
3 Moderate hypomimia; lips parted some of the time.
3 Marked excess of saliva with some drooling.
4 Marked or fixed facies with severe or complete loss of expression;
4 Marked drooling, requires constant tissues or handkerchief.
lips parted ¼ inch or more.
2. Swallowing
0 Normal.
1 Rare choking.
2 Occasional choking.
3 Requires soft food.
4 Requires NG tube or gastrostomy feeding.
5.Cough
0 Normal.
1 Slightly impaired.
2 Markedly impaired.
3 Weak.
4 Impossible or artificial measures required (suctioning,
tracheotomy).
3. Speech (motor examination)
0 Normal.
1 Slight loss of expression, dictation and/or volume.
2 Monotone, slurred but understandable; moderately impaired.
3 Marked impairment, difficult to understand.
4 Unintelligible.
6. Speech (ADL)
0 Normal.
1 Mildly affected. No difficulty being understood.
2 Moderately affected. Sometimes asked to repeat statements.
3 Severely affected, Frequently asked to repeat statements.
4 Unintelligible most of the time.
ADL / MOBILITY (interview with patient or caregiver)
1. Handwriting
0 Normal.
1 Slightly slow or small.
2 Moderately slow or small ; all words are legible.
3 Severely affected; not all words are legible.
4 The majority of words are not legible.
5. Turning in bed and adjusting bed clothes
0 Normal.
1 Somewhat slow and clumsy, but no help needed
2 Can turn alone or adjust sheets, but with great difficulty.
3 Can initiate, but not turn or adjust sheets alone.
4 Helpless.
2. Cutting food and handling utensils
6. Falling unrelated to freezing
0 Normal.
0 None.
1 Somewhat slow and clumsy, but no help needed.
2 Can cut most foods, although clumsy and slow; some help needed.1 Rare falling.
2 Occasional falls, less than once per day.
3 Food must be cut by someone, but can still feed slowly.
3 Falls an average of once a day.
4 Needs to be fed.
4 Falls more than once daily or wheelchair bound due to falls
9 Wheelchair bound due to other reason (accident …),
3. Dressing
specify : __________________________
0 Normal.
1 Somewhat slow, but no help needed.
7. Freezing when walking
2 Occasional assistance with buttoning, getting arms in sleeves.
0 None.
3 Considerable help required, but can do some things alone.
1 Rare freezing when walking; may have start-hesitation.
4 Helpless.
2 Occasional freezing when walking.
3 Frequent freezing. Occasional falls from freezing
4. Hygiene
4 Frequent falls from freezing or wheelchair bound due to freezing
0 Normal.
9 If not assessable, specify why : (example: MSA Cerebellar type)
1 Somewhat slow, but no help needed.
2 Needs help to shower or bathe ; or very slow in hygiene care.
3 Requires assistance for washing, brushing teeth, combing hair,
going to bathroom.
4 Foley catheter or other mechanical aids.
8. Walking
0 Normal.
1 Mild difficulty. May not swing arms or may tend to drag legs.
2 Moderate difficulty, but requires little or no assistance.
3 Severe disturbances or walking, requiring assistance.
4 Cannot walk at all, even with assistance.
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NNIPPS-PPS 3/6
TREMOR
1. Tremor/ADL
0 Absent.
1 Slight and infrequently present.
2 Moderate, bothersome to patient
3 Severe ; interferes with many activities
4 Marked ; interferes with most activities.
2. Face, lips, chin tremor
0 Absent.
1 Slight and infrequently present.
2 Mild in amplitude and persistent ; or moderate in amplitude, but
only intermittently present.
3 Moderate in amplitude and present most of the time.
4 Marked in amplitude and present most of the time.
3. Right hand tremor at rest
0 Absent.
1 Slight and infrequently present
2 Mild in amplitude and persistent ; or moderate in amplitude, but
only intermittently present.
3 Moderate in amplitude and present most of the time.
4 Marked in amplitude and present most of the time.
4. Left hand tremor at rest
0 Absent.
1 Slight and infrequently present.
2 Mild in amplitude and persistent ; or moderate in amplitude, but
only intermittently present.
3 Moderate in amplitude and present most of the time.
4 Marked in amplitude and present most of the time.
5. Right foot tremor at rest
0 Absent.
1 Slight and infrequently present.
2 Mild in amplitude and persistent ; or moderate in amplitude, but
only intermittently present.
3 Moderate in amplitude and present most of the time.
4 Marked in amplitude and present most of the time.
6. Left foot tremor at rest
0 Absent.
1 Slight and infrequently present.
2 Mild in amplitude and persistent ; or moderate in amplitude, but
only intermittently present.
3 Moderate in amplitude and present most of the time.
4 Marked in amplitude and present most of the time.
7. Action or postural tremor of right hand
0 Absent.
1 Slight ; present with action.
2 Moderate in amplitude, present with action.
3 Moderate in amplitude with posture holding as well as action.
4 Marked in amplitude ; interferes with feeding.
8. Action or postural tremor of left hand
0 Absent.
1 Slight ; present with action.
2 Moderate in amplitude, present with action.
3 Moderate in amplitude with posture holding as well as action.
4 Marked in amplitude; interferes with feeding.
RIGIDITY
1. Neck rigidity
0 Absent.
1 Slight or detectable only when activated by mirror or other
movement.
2 Mild to moderate.
3 Marked, but full range of motion easily achieved.
4 Severe, range of motion achieved with difficulty.
2. Right upper extremity rigidity
0 Absent.
1 Slight or detectable only when activated by mirror or other
movement.
2 Mild to moderate.
3 Marked, but full range of motion easily achieved.
4 Severe, range of motion achieved with difficulty.
3. Left upper extremity rigidity
0 Absent.
1 Slight or detectable only when activated by mirror or other
movement.
2 Mild to moderate.
3 Marked, but full range of motion easily achieved.
4 Severe, range of motion achieved with difficulty.
4. Right lower extremity rigidity
0 Absent.
1 Slight or detectable only when activated by mirror or other
movement.
2 Mild to moderate.
3 Marked, but full range of motion easily achieved.
4 Severe, range of motion achieved with difficulty.
5. Left lower extremity rigidity
0 Absent.
1 Slight or detectable only when activated by mirror or other
movement.
2 Mild to moderate.
3 Marked, but full range of motion easily achieved.
4 Severe, range of motion achieved with difficulty.
MYOCLONUS
1. At rest
0 Absent.
1 Mild.
2 Obvious.
3 Disabling.
4 Severe.
2. In action
0 Absent.
1 Mild.
2 Obvious.
3 Disabling.
4 Severe.
3.With stimulation
0 Absent.
1 Mild.
2 Obvious.
3 Disabling.
4 Severe.
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NNIPPS-PPS 4/6
LIMB BRADYKINESIA
1. Right fingers taps
5. Rapid alternating movements of the right hand
0 Normal.
0 Normal.
1 Mild slowing and/or reduction in amplitude.
1 Mild slowing and/or reduction in amplitude.
2 Moderately impaired. Definite and early fatiguing. May have
2 Moderately impaired. Definite and early fatiguing. May have
occasional arrests in movement.
occasional arrests in movement.
3 Severely impaired. Frequent hesitation in initiating movements or 3 Severely impaired. Frequent hesitation in initiating movements or
arrests in ongoing movement.
arrests in ongoing movement.
4 Can barely perform the task.
4 Can barely perform the task.
2. Left fingers taps
0 Normal.
1 Mild slowing and/or reduction in amplitude.
2 Moderately impaired. Definite and early fatiguing. May have
occasional arrests in movement.
3 Severely impaired. Frequent hesitation in initiating movements or
arrests in ongoing movement.
4 Can barely perform the task.
6. Rapid alternating movements of the left hand
0 Normal.
1 Mild slowing and/or reduction in amplitude.
2 Moderately impaired. Definite and early fatiguing. May have
occasional arrests in movement.
3 Severely impaired. Frequent hesitation in initiating movements or
arrests in ongoing movement.
4 Can barely perform the task.
3. Right hand movements
0 Normal.
1 Mild slowing and/or reduction in amplitude.
2 Moderately impaired. Definite and early fatiguing. May have
occasional arrests in movement.
3 Severely impaired. Frequent hesitation in initiating movements or
arrests in ongoing movement.
4 Can barely perform the task.
7. Right Leg agility
0 Normal.
1 Mild slowing and/or reduction in amplitude.
2 Moderately impaired. Definite and early fatiguing. May have
occasional arrests in movement.
3 Severely impaired. Frequent hesitation in initiating movements or
arrests in ongoing movement.
4 Can barely perform the task.
4. Left hand movements
0 Normal.
1 Mild slowing and/or reduction in amplitude.
2 Moderately impaired. Definite and early fatiguing. May have
occasional arrests in movement.
3 Severely impaired. Frequent hesitation in initiating movements or
arrests in ongoing movement.
4 Can barely perform the task.
8. Left Leg agility
0 Normal.
1 Mild slowing and/or reduction in amplitude.
2 Moderately impaired. Definite and early fatiguing. May have
occasional arrests in movement.
3 Severely impaired. Frequent hesitation in initiating movements or
arrests in ongoing movement.
4 Can barely perform the task.
AXIAL BRADYKINESIA
1. Arising from chair
4. Gait
0 Normal.
0 Normal.
1 Slow or may need more than one attempt.
1 Walks slowly, may shuffle with short steps, but no festination or
2 Pushes self up from arms of seat.
propulsion.
3 Tends to fall back and may have to try more than one time, but can2 Walks with difficulty, but requires little or no assistance; may have
get without help.
some festinations, short steps, or propulsion.
4 Unable to arise without help or cannot stand.
3 Severe disturbance of gait, requiring assistance.
4 Cannot walk at all, even with assistance.
2. Sitting down
0 Normal.
5. Postural stability
1 Slightly stiff or awkward.
0 Normal.
2 Easily positions self before chair, but descent into chair is
1 Retropulsion, but recovers unaided.
uncontrolled.
2 Absence of postural response; would fall if not caught by examiner
3 Has difficulty finding chair behind him/her and descent is
3 Very unstable, tends to lose balance spontaneously.
uncontrolled.
4 Unable to stand without assistance.
4 Unable to test because of severe postural instability.
6. Body bradykinesia and hypokinesia
0 None.
3. Posture
0 Normal erect.
1 Minimal slowness, giving movement a deliberate character; could be
1 Not quite erect, slightly stooped posture; could be normal for an normal for some persons. Possible reduced amplitude.
older person.
2 Mild degree of slowness and poverty of movement which is definitely
2 Moderately stooped posture, definitely abnormal; can be slightly abnormal. Alternatively, some reduced amplitude.
leaning to one side.
3 Moderate slowness, poverty or small amplitude of movements.
3 Severely stooped posture with kyphosis; can be moderately leaning4 Marked slowness, poverty or small amplitude of movements
to one side.
4 Marked flexion with extreme abnormality of posture.
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NNIPPS-PPS 5/6
OCULOR MOTOR FUNCTION
1. Speed of voluntary upward saccades
0 Normal speed.
1 Mild slowness.
2 Definite slowness.
3 Severe slowness or no saccades.
2. Amplitude of voluntary upward saccades
0 Not hypometric.
1 Mild limitation.
2 Moderate limitation.
3 Severe limitation.
3. Speed of voluntary downward saccades
0 Normal speed.
1 Mild slowness.
2 Definite slowness.
3 Severe slowness or no saccades.
4. Amplitude of voluntary downward saccades
0 Not hypometric.
1 Mild limitation.
2 Moderate limitation.
3 Severe limitation.
AXIAL DYSTONIA
1. Face
0 Absent.
1 Subtle or present : only when activated by other movement.
2 Obvious but not continuous.
3 Only partial range of motion possible.
4 Continuous and disabling.
2. Neck
0 Absent
1 Subtle or present : only when activated by other
movement.
2 Obvious but not continuous
3 Only partial range of motion possible.
4 Continuous and disabling.
3. Trunk
0 Absent.
1 Subtle or present : only when activated by other movement.
2 Obvious but not continuous.
3 Only partial range of motion possible.
4 Continuous and disabling.
LIMB DYSTONIA
5. Speed of voluntary left and right saccades
0 Normal speed.
1 Mild slowness.
2. Definite slowness.
3 Severe slowness or no saccades.
6. Amplitude of voluntary left and right saccades
0 Not hypometric.
1 Mild limitation.
2 Moderate limitation.
3 Severe limitation.
1. Left Upper limb
0 Absent.
1 Subtle or present : only when activated by other movement.
2 Obvious but not continuous.
3 Only partial range of motion possible.
4 Continuous and disabling.
2. Right Upper limb
0 Absent.
1 Subtle or present : only when activated by other movement.
2 Obvious but not continuous.
3 Only partial range of motion possible.
4 Continuous and disabling.
7.Eyelid dysfunction
0 None.
1 Mild inhibition of opening or closing or mild blepharospasm :
3. Left Lower limb
no visual disability.
0 Absent.
2 Moderate lid-opening inhibition or blepharospasm causing
1 Subtle or present : only when activated by other movement.
partial visual disability.
3 Functional blindness or near-blindness because of involuntary 2 Obvious but not continuous.
3 Only partial range of motion possible.
eyelid closure.
4 Continuous and disabling.
4. Right lower limb
0 Absent.
1 Subtle or present : only when activated by other movement.
2 Obvious but not continuous.
3 Only partial range of motion possible.
4 Continuous and disabling.
PYRAMIDAL SIGNS
1. Masseter or jaw reflex
0 Normal.
1 Exaggerated.
3. Deep tendon reflex Biceps, triceps, brachoradialis, quadriceps,
triceps surae
0 All Normal or any decreased.
1 Abnormally brisk (any abnormally brisk).
2. Babinski sign
0 Negative (score the bilateral flexion).
1 Mute or equivocal (bilateral).
2 Extension (either side).
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NNIPPS-PPS 6/6
CEREBELLAR FUNCTION
ORTHOSTATIC SYMPTOMS (interview)
1. Ataxia
1. In the past 12 months (6 months at follow-up): History of
0 No abnormality.
faintness or dizziness soon after standing-up from a sitting or
1 Some abnormalities without disability.
lying position
2 Mild ataxia of one limb.
0 No
3 Moderate ataxia of trunk, walking or 2 limbs, or severe ataxia 1 Yes
of one limb.
IF YES COMPLETE THE FOLLOWING (if no subsequent
4 Moderate ataxia of 3 to 4 limbs, or severe ataxia of 2 limbs. ratings = 0)
5 Severe ataxia of 3 to 4 limbs, or severe disability to coordinate 1. How frequent did the patient get these symptoms ?
movement related to ataxia.
1 Rarely, once a week or less.
1 Occasionally, several times a week.
3 Frequently, at least once a day.
2. Gaze-evoked nystagmus
0 Normal.
4 Almost always, several times a day.
1 Transient.
2 Persistent but moderate.
2. How does the patient rate the severity of these symptoms ?
3 Persistent and severe.
1 Mild.
2 Moderate.
3. Knee- tibia test : Lowering of heel in continuous axis
3 Severe.
LEFT LEG
0 Normal.
3. In the past 12 months (6 months at follow-up): how often did
1 The movement is decomposed in several phases, without real the patient end-up fainting soon after standing-up
jerks, or abnormally slow.
0 Never.
2 Lowering jerkily in the axis.
1 Once.
3 Lowering jerkily with lateral movements.
2 Twice.
4 Lowering jerkily with extremely strong lateral movements. 3 Three times.
9 Test impossible, specify why : __________________
4 Four times.
5 Five or more times.
RIGHT LEG
0 Normal.
1 The movement is decomposed in several phases, without real
URINARY SYMPTOMS (interview)
jerks, or abnormally slow.
1. In the past 12 months (6 months at follow-up): did the
2 Lowering jerkily in the axis.
patient ever leak urine or lost cotrol of bladder function ?
3 Lowering jerkily with lateral movements.
4 Lowering jerkily with extremely strong lateral movements or 0 None or few drops less than once daily.
1 A few drops staining clothes daily.
tests impossible.
9 Test impossible, specify why : __________________
2 Large amounts, but only when asleep, no pad required during
day.
4. Finger to nose test (decomposition and dysmetria)
3 Occasional large amounts in daytime : pad required.
LEFT ARM
4 Consistent, requiring diaper or catheter awake and asleep.
0 No trouble.
1 Oscillating movement without decomposition of the
2. In the past 12 months (6 months at follow-up): did the
movement.
patient experience difficulties passing urine ?
2 Segmented movement in 2 phases and/or moderate dysmetria
0 Never.
in reaching nose.
3 Segmented movement in more than 2 phases or considerable 1 Occasionally.
2 Frequently.
dysmetria in reaching nose.
3 Constantly or catheter in site.
4 Dysmetria preventing the patient from reaching nose.
9 Test impossible, specify why : __________________
3. In the past 12 months(6 months at follow-up): did the patient
ever experience trouble completely emptying bladder ?
RIGHT ARM
0 Never.
0 No trouble.
1 Oscillating movement without decomposition of the
1 Occasionally.
movement.
2 Frequently.
2 Segmented movement in 2 phases and/or moderate dysmetria 3 Constantly or catheter in site.
in reaching nose.
3 Segmented movement in more than 2 phases or considerable
dysmetria in reaching nose.
4 Dysmetria preventing the patient from reaching nose.
9 Test impossible, specify why : __________________
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